Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets

Background Hand involvement is an early manifestation of systemic sclerosis (SSc), culprit of diagnosis and classification, and recognised major driver of disability. Impairment of hand function burdens both limited and diffuse cutaneous subsets and therefore could be targeted as ‘basket’ endpoint i...

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Main Authors: Lorraine Green, Ariane L Herrick, Michelle Wilson, Francesco Del Galdo, Enrico De Lorenzis, Philip Helliwell, Benazir Saleem, Emma Derrett-Smith, Begonya Alcacer-Pitarch, Karen Douglas, Vishal Kakkar, Chris Denton, Theresa Barnes, Stefano Di Donato, Muhammad Nisar, Catherine Morley
Format: Article
Language:English
Published: BMJ Publishing Group 2024-02-01
Series:RMD Open
Online Access:https://rmdopen.bmj.com/content/10/1/e003216.full
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author Lorraine Green
Ariane L Herrick
Michelle Wilson
Francesco Del Galdo
Enrico De Lorenzis
Philip Helliwell
Benazir Saleem
Emma Derrett-Smith
Begonya Alcacer-Pitarch
Karen Douglas
Vishal Kakkar
Chris Denton
Theresa Barnes
Stefano Di Donato
Muhammad Nisar
Catherine Morley
author_facet Lorraine Green
Ariane L Herrick
Michelle Wilson
Francesco Del Galdo
Enrico De Lorenzis
Philip Helliwell
Benazir Saleem
Emma Derrett-Smith
Begonya Alcacer-Pitarch
Karen Douglas
Vishal Kakkar
Chris Denton
Theresa Barnes
Stefano Di Donato
Muhammad Nisar
Catherine Morley
author_sort Lorraine Green
collection DOAJ
description Background Hand involvement is an early manifestation of systemic sclerosis (SSc), culprit of diagnosis and classification, and recognised major driver of disability. Impairment of hand function burdens both limited and diffuse cutaneous subsets and therefore could be targeted as ‘basket’ endpoint in SSc. Nevertheless, its natural history in current standard of care is not well characterised, limiting the design of targeted trials. The aim of this study is to describe prevalence, natural history and clinical factors associated with hand function deterioration in a longitudinal, multicentre, observational SSc cohort.Methods Hand function was captured through the validated Cochin Hand Function Scale in patients consecutively enrolled in a multicentre observational study and observed over 24 months. Minimal clinically important differences and patient acceptable symptom state were analysed as previously described.Results Three hundred and ninety-six consecutive patients were enrolled from 10 centres; 201 with complete follow-up data were included in the analysis. Median (IQR) disease duration was 5 (2–11) years. One hundred and five (52.2%) patients reported clinically significant worsening. Accordingly, the proportion of patients reporting unacceptable hand function increased over 2 years from 27.8% to 35.8% (p<0.001). Least absolute shrinkage and selection operator analysis identified male gender, disease subset, Raynaud’s Condition Score, tenosynovitis and pain, as some of the key factors associated with worsening hand involvement.Conclusions Hand function deteriorates over time in more than 50% of SSc patients despite available therapies. The analysis of factors associated with hand function worsening supports the involvement of both inflammation, vascular and fibrotic processes in hand involvement, making it a hallmark clinical manifestation of SSc. Our data are poised to inform the design of intervention studies to target this major driver of disability in SSc.
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spelling doaj-art-7b56cfd98b3d45acba6ab68e802e796f2025-08-20T03:48:31ZengBMJ Publishing GroupRMD Open2056-59332024-02-0110110.1136/rmdopen-2023-003216Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsetsLorraine Green0Ariane L Herrick1Michelle Wilson2Francesco Del Galdo3Enrico De Lorenzis4Philip Helliwell5Benazir Saleem6Emma Derrett-Smith7Begonya Alcacer-Pitarch8Karen Douglas9Vishal Kakkar10Chris Denton11Theresa Barnes12Stefano Di Donato13Muhammad Nisar14Catherine Morley15NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UKCentre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust,Manchester Academic Health Science Centre, Manchester, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKDepartment of Rheumatology, St. Luke’s Hospital, Bradford Teaching Hospitals NHS Foundation Trust, Bradford, UKDepartment of Rheumatology, York Teaching Hospital NHS Foundation Trust, York, UKDepartment of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKDepartment of Rheumatology, The Dudley Group NHS Foundation Trust, Dudley, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKCentre for Rheumatology, University College London, London, UKDepartment of Rheumatology, Countess of Chester Hospital NHS Foundation Trust, Liverpool, UKLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKDepartment of Rheumatology, Luton & Dunstable University Hospital NHS Foundation Trust, Luton, UKDepartment of Rheumatology, South Tyneside and Sunderland NHS Foundation Trust, Sunderland, UKBackground Hand involvement is an early manifestation of systemic sclerosis (SSc), culprit of diagnosis and classification, and recognised major driver of disability. Impairment of hand function burdens both limited and diffuse cutaneous subsets and therefore could be targeted as ‘basket’ endpoint in SSc. Nevertheless, its natural history in current standard of care is not well characterised, limiting the design of targeted trials. The aim of this study is to describe prevalence, natural history and clinical factors associated with hand function deterioration in a longitudinal, multicentre, observational SSc cohort.Methods Hand function was captured through the validated Cochin Hand Function Scale in patients consecutively enrolled in a multicentre observational study and observed over 24 months. Minimal clinically important differences and patient acceptable symptom state were analysed as previously described.Results Three hundred and ninety-six consecutive patients were enrolled from 10 centres; 201 with complete follow-up data were included in the analysis. Median (IQR) disease duration was 5 (2–11) years. One hundred and five (52.2%) patients reported clinically significant worsening. Accordingly, the proportion of patients reporting unacceptable hand function increased over 2 years from 27.8% to 35.8% (p<0.001). Least absolute shrinkage and selection operator analysis identified male gender, disease subset, Raynaud’s Condition Score, tenosynovitis and pain, as some of the key factors associated with worsening hand involvement.Conclusions Hand function deteriorates over time in more than 50% of SSc patients despite available therapies. The analysis of factors associated with hand function worsening supports the involvement of both inflammation, vascular and fibrotic processes in hand involvement, making it a hallmark clinical manifestation of SSc. Our data are poised to inform the design of intervention studies to target this major driver of disability in SSc.https://rmdopen.bmj.com/content/10/1/e003216.full
spellingShingle Lorraine Green
Ariane L Herrick
Michelle Wilson
Francesco Del Galdo
Enrico De Lorenzis
Philip Helliwell
Benazir Saleem
Emma Derrett-Smith
Begonya Alcacer-Pitarch
Karen Douglas
Vishal Kakkar
Chris Denton
Theresa Barnes
Stefano Di Donato
Muhammad Nisar
Catherine Morley
Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
RMD Open
title Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
title_full Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
title_fullStr Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
title_full_unstemmed Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
title_short Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets
title_sort clinical trajectories of hand function impairment in systemic sclerosis an unmet clinical need across disease subsets
url https://rmdopen.bmj.com/content/10/1/e003216.full
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